scholarly journals Carcinoembryonic antigen levels are increased with pulmonary output in pulmonary hypertension due to congenital heart disease

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052096437
Author(s):  
Yang Zi-yang ◽  
Zhao Kaixun ◽  
Luo Dongling ◽  
Yin Zhou ◽  
Zhou Chengbin ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Carcinoembryonic Antigen ◽  
Congenital Heart ◽  
Pulmonary Artery Hypertension ◽  
Group A ◽  
Serum Cea ◽  
Pulmonary Arterial ◽  
Group B

Objective Pulmonary artery hypertension (PAH) is a severe complication of congenital heart disease (CHD). Monitoring of pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) is essential during follow-up. This retrospective study aimed to examine carcinoembryonic antigen (CEA) as an additional marker for evaluation by investigating the correlation between CEA levels and hemodynamics in CHD-PAH. Methods Seventy-six patients with CHD-PAH (mean PAP [mPAP] >25 mmHg and PVR >3 Wood units, group A), 71 patients with CHD and pulmonary hypertension (CHD-PH, mPAP >25 mmHg and PVR ≤3 Wood units, group B), and 102 patients with CHD without PH (mPAP ≤25 mmHg, group C) were enrolled. Serum CEA levels and the relationships between CEA levels and hemodynamic data were assessed. Results Mean serum CEA levels were 1.99±1.61, 2.44±1.82, and 1.58±1.07 ng/mL, mPAP was 58.66±20.21, 30.2±4.83, and 17.31±4.51 mmHg, and PVR was 10.12±7.01, 2.19±0.56, and 2.2±1.1 Wood units in groups A, B, and C, respectively. Mean pulmonary output (PO) was 7.24±3.07, 15.79±5.49, 10.18±4.72 L/minute, respectively. CEA levels were positively correlated with PO and negatively correlated with PVR in all of the patients. Conclusion CEA levels are increased with PO and decreased with PVR in CHD-PH.

scholarly journals Efficacy of citrulline supplementation to decrease the risk of pulmonary hypertension after congenital heart disease surgery. A local experience.

2020 ◽  
Vol 77 (4) ◽  
pp. 249-253
Author(s):  
Silene Silvera Ruiz ◽  
Carola L Grosso ◽  
Margot Tablada ◽  
Marcelo Cabrera ◽  
Raquel Dodelson de Kremer ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
Reference Level ◽  
Plasma Citrulline ◽  
Post Surgery ◽  
Group A ◽  
Group B

Pulmonary hypertension (PH) is a major cause of morbi-mortality among patients with congenital heart disease (CHD) and also a potentially severe complication after surgical repair. Oral citrulline, a precursor to NO synthesis, is safe and efficacious for decreasing the risk of postoperative PH. Objective: The aim of the present study was to investigate in pediatric patients the changes of plasma citrulline, arginine, homocysteine and nitric oxide (NO) metabolites and pulmonary artery pressures (PAP) pre-post cardiac surgery in order to describe our population status with regard to the risk of pulmonary hypertension and look for potential biomarkers for early detection and treatment. Main results/Discussion: 16 Argentine pediatric patients with CHD undergoing cardiopulmonary bypass were randomized in two groups: (A) with and (B) without perioperative citrulline supplementation. We found that plasma citrulline median levels before surgery were lower in both groups respect to referential values, probably due to the poor nutritional status of our patients; only group A surpassed post-surgery the minimum recommended level to avoid PH. Furthermore, none of the patients in group A showed mean PAP higher than 20 mmHg, whereas in group B, 67% of the measurements were ≥ than the reference level. Conclusions: We reaffirm that citrulline supplementation it is effective in reducing postoperative pulmonary hypertension and biomarkers could evidence patient status as a translational medicine application.

scholarly journals Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Constantine ◽  
K Dimopoulos ◽  
R Condliffe ◽  
P Clift ◽  
G Chaplin ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Free Survival ◽  
Paediatric Cohort ◽  
Group A ◽  
Pulmonary Arterial ◽  
Group B ◽  
Group D ◽  
Over Time

Abstract Background/Introduction Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) in children and is associated with significant morbidity and mortality. The impact of different phenotypes on management and survival remains unclear. Purpose To examine the clinical features, management and outcomes of paediatric PAH-CHD patients based on the 20-year experience of the UK National Paediatric Pulmonary Hypertension (PH) Service. Methods Consecutive PAH-CHD patients entering the service between January 2001 and January 2021 were included and classified into: Eisenmenger syndrome (ES, group A), PAH related to a significant systemic-pulmonary shunt (group B), PAH with small or co-incidental CHD (group C) and PAH following defect repair (group D). Incident patients (without pre-existing PAH) were included in survival analysis. Results Of the overall PH paediatric cohort of 1104 patients, 819 (74.2%) had co-existing CHD and 354 (32.1%) patients received a diagnosis of PAH-CHD: 57.1% female, median [IQR] 4.6 [1.7–10.9] years. Group D PAH-CHD was the commonest subgroup, accounting for 36%, while the least frequent subtype was group C (14%). Group A and group B PAH-CHD represented 26% and 24%, respectively. Down syndrome was present in over one third (122, 34.5%) of PAH-CHD patients and was more commonly associated with ES (p=0.02). PAH therapy was started in 79.9% of PAH-CHD patients. At the end of follow-up, patients with group C PAH-CHD were more likely to be on combination therapy than any other group (64.6% vs. 28.4%, p<0.0001). Prostanoid therapy was used in a minority (11%) of patients. The subgroup distribution of PAH-CHD at diagnosis changed from the early (2000–2005) to late (2015–2020) period (Figure 1). The proportion of ES patients decreased from 43.4% to 14.6% of PAH-CHD (p<0.0001). The proportion of group B PAH-CHD patients increased (9.4% vs. 33.3%, p<0.0001), with the majority (59.3%) deemed “operable” on specialist assessment. There was a trend for an increase in repaired PAH-CHD patients between the early and late era (31.1% vs. 43.8%, p=0.09). Transplant-free survival in PAH-CHD was 90.9% (95% CI: 87.8–94%) at 1 year, 77.9% (95% CI: 73.1–83.1%) at 5 years, and 74.9% (95% CI: 69.6–80.7%) at 10 years (Figure 2). Group C PAH-CHD had a lower transplant-free survival than the other 3 groups (HR 2.54, 95% CI: 1.51–4.28, p=0.0005). There was no difference in outcome between group A and group D PAH-CHD (HR 1.19, 95% CI: 0.62–2.28, p=0.6). Conclusions Repaired PAH-CHD, not ES, was the most common subtype in this large paediatric cohort. Over time, there were fewer ES patients and more “operable” patients with left-right shunts, suggesting an improvement in early diagnosis and management. Despite widespread use of PAH therapy, PAH-CHD remains a life-limiting disease with the poorest outcomes in PAH with co-incidental CHD. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Dr Constantine received a personal PhD fellowship grant (CHAMPION PhD Fellowship) Figure 1 Figure 2

Pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 781-784
Author(s):  
Shahin Moledina ◽  
Bejal Pandya
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Careful Assessment

Congenital heart disease is a major cause of pulmonary arterial hypertension (PAH) and this can largely be prevented by early repair. PAH in the presence of systemic-to-pulmonary communication, leads to shunt reversal and cyanosis, with multiple systemic consequences (Eisenmenger syndrome). Congenital heart disease patients with PAH are vulnerable and are at high risk from non-cardiac surgery, pregnancy, and inappropriate medical treatment (e.g. excessive venesection). Survival is reduced, but is better than in idiopathic PAH. Recommendations for surgery should be based on careful assessment by experts. Modern PAH pharmacotherapy is showing promise in improving quality of life.

scholarly journals COMPARISON OF INTRANASAL DEXMEDETOMIDINE V/S MIDAZOLAM AS A PREMEDICATION IN CHIDREN WITH CONGENITAL HEART DISEASE UNDERGOING CARDIAC SURGERY

2021 ◽  
Vol 9 (01) ◽  
pp. 814-822
Author(s):  
Sanjay Kumar Morwal ◽  
Ajit Singh ◽  
C.K. Vyas ◽  
Neelu Sharma
Keyword(s):  
Congenital Heart Disease ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Arterial Blood ◽  
Sedative Effects ◽  
Group A ◽  
Child Separation ◽  
Group B ◽  
Intranasal Midazolam

Background: The intranasal route is a reliable way to administer preanaesthetics and sedatives to children. The aim of present study was to compare the anxiolytic and sedative effects of intranasal dexemedetomidine and midazolam as a premedication in children with congenital heart disease undergoing cardiac surgery.. Patients and Methods: Fifty chidrens 2-12 years of age group of either sex with congenital heart disease undergoing cardiac surgery were randomly allocated into two groups. Group A-(n=25) - received intranasal dexmedetomidine as premedication.(0.1 µg/kg diluted in 2ml NS ) Group B- (n=25)- received intranasal midazolam as premedication. (0.2 mg/kg diluted in 2ml NS) Heart rate, mean arterial blood pressure and oxygen saturation were monitored upto30 minute after drug administration. The sedation score, anxiety score and child separation score were recorded until the child taken to the OR. The postoperative agitation score was also observed. Results and Conclusion: Premedication with intranasal dexmedetomidine attained significant and satisfactory sedation with better parental separation and lower anxiety levels without any adverse effects as compared with intranasal midazolam in children with congenital heart disease undergoing cardiac surgery.

Pulmonary hypertension

2020 ◽  
pp. 3695-3710
Author(s):  
Nicholas W. Morrell
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Congenital Heart ◽  
Heart Function ◽  
Gene Encoding ◽  
Pulmonary Arterial

Symptoms of unexplained exertional breathlessness or symptoms out of proportion to coexistent heart or lung disease should alert the clinician to the possibility of pulmonary hypertension, and the condition should be actively sought in patients with known associated conditions, such as scleroderma, hypoxic lung disease, liver disease, or congenital heart disease. Heterozygous germ-line mutations in the gene encoding the bone morphogenetic protein type II receptor (BMPR2) are found in over 70% of families with pulmonary arterial hypertension. Pulmonary hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest, and may be due to increased pulmonary vascular resistance (e.g. pulmonary arterial hypertension), increased transpulmonary blood flow (e.g. congenital heart disease), or increased pulmonary venous pressures (e.g. mitral stenosis). Exercise tolerance and survival in pulmonary hypertension is ultimately related to indices of right heart function, such as cardiac output.

scholarly journals Diagnosis of Neonatal Congenital Heart Disease: A Combination of Heart Murmur, SpO2 Abnormality, Tachypnea, and Extracardiac Malformations

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Kai Chen ◽  
Jiao Wang ◽  
Huihui Zhou ◽  
Xiang Huang
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Murmur ◽  
Heart Murmurs ◽  
Group A ◽  
Postnatal Outcome ◽  
Group B ◽  
Group D ◽  
Youden’S Index

Congenital heart disease (CHD) is one of the commonest congenital malformations that are mostly asymptomatic at birth, which challenges the diagnosis of neonatal CHD. An early accurate prenatal diagnosis will give parents a choice, as well as the opportunity to plan the delivery and improve the postnatal outcome. The purpose of the study is to evaluate the value of heart murmurs, SpO2 abnormalities, tachypnea, and extracardiac malformations in screening neonatal CHD. All 4500 newborns in the obstetrics department of our hospital from January 2019 to January 2020 are selected as study subjects. Newborns were grouped according with the presence of heart murmurs, tachypnea, transdermal SpO 2 < 95 % , and extracardiac malformations alone or in combination (≥3). Patients with murmur, tachypnea, and abnormal SpO2 were assigned into group A, those with murmur, tachypnea, and extracardiac malformations into group B, those with murmurs, SpO2, and extracardiac malformations into group C, those with SpO2, tachypnea, and extracardiac malformations into group D, and those with all four into group E. The color echocardiography identified 65 children with CHD (1.4%) among the included 4,500 newborns. When murmur, tachypnea, abnormal SpO2, and extracardiac malformation were independently used to diagnose CHD, the sensitivity ranged from 30.68% to 51.26%, with specificity ranging from 47.36% to 82.65% and Youden’s index (YI) ranging from 0.13 to 0.36. When murmur, tachypnea, abnormal SpO2, extracardiac malformation were together used to diagnose CHD, 91.23% sensitivity, 95.26 specificity, and 0.91 YI were observed. In conclusion, a combination of four indicators, murmur, tachypnea, abnormal SpO2, and extracardiac malformation yielded good performance in diagnosing neonatal CHD.

Changes in ET-1, Plasma Neuropeptide Y, and CGRP in Child Patients With Congenital Heart Disease Complicated With Pulmonary Hypertension Before and After Operation

2020 ◽  
Vol 60 (1) ◽  
pp. 56-63
Author(s):  
Zheng Liu ◽  
Mingming Zhang ◽  
Qiang Huo ◽  
Tao Zhu
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Neuropeptide Y ◽  
Congenital Heart ◽  
Control Group ◽  
Child Patients ◽  
Before And After ◽  
Pulmonary Arterial ◽  
Positive Correlations

This study aims to explore the changes in endothelin-1 (ET-1), plasma neuropeptide Y, and calcitonin gene–related peptide (CGRP) in child patients before and after operation. A total of 80 child patients with congenital heart disease (CHD) complicated with pulmonary hypertension (PH) were enrolled and divided into control group (n = 40, conservative treatment for various reasons) and observation group (n = 40, active preoperative preparation and timely operative intervention) according to different treatments. There were positive correlations between systolic pulmonary arterial pressure (sPAP) and ET-1, plasma neuropeptide Y, while negative correlation between sPAP and CGRP. In conclusion, our data demonstrate that the levels of ET-1, plasma neuropeptide Y, and CGRP in PH-CHD were significantly changed after interventions, which provides new leads as alternative biomarkers to assess the efficacy of treatments against PH-CHD.

Selexipag use for paediatric pulmonary hypertension: a single centre report focussed on congenital heart disease patients

2021 ◽  
pp. 1-3
Author(s):  
Alvaro Lafuente-Romero ◽  
Alejandro Rodriguez Ogando
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Poor Prognosis ◽  
Receptor Agonist ◽  
Congenital Heart ◽  
Complex Disease ◽  
Pulmonary Artery Hypertension ◽  
Single Centre ◽  
Prostacyclin Receptor

Abstract Pulmonary hypertension is a rare and complex disease with poor prognosis. Paediatric cases are infrequent and usually associated with congenital heart disease. Management is problematical due to the limited therapy available and poor evidence of efficacy. Recently a new medication, selexipag (UptraviR), a prostacyclin receptor agonist, has been approved for the treatment of pulmonary artery hypertension in adults. We report our experience using selexipag in four paediatric patients with pulmonary hypertension associated with congenital heart disease.

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